Cutaneous Lymphomas — MCQs
Mycosis fungoides primarily involves which type of immune cell?
Identify the diagnosis based on the dermatology immunofluorescence (IF) image provided.
Which of the following statements about Mycosis fungoides is not true?
Which marker is commonly associated with positivity in follicular lymphoma?
A 22-year-old woman presents with painless cervical lymphadenopathy, night sweats, and generalized pruritus. An enlarged cervical lymph node is removed for diagnosis, which shows broad bands of fibrosis on cut surface and histologically contains a mixture of cells, including lymphocytes, histiocytes, eosinophils, plasma cells, and scattered large cells with prominent nucleoli. Which of the following is the most likely condition?
Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen
Pautrier's microabscess is a histological feature of which disease?
What is the dermatological sign associated with carcinoma of the stomach?
What is the most common malignancy found in Marjolin's ulcer?
What is a rodent ulcer?
Cutaneous Lymphomas Indian Medical PG Practice Questions and MCQs
Question 1: Mycosis fungoides primarily involves which type of immune cell?
- A. NK cells
- B. B lymphocytes
- C. Plasma cells
- D. T lymphocytes (Correct Answer)
Explanation: ***CD4+ T Cells*** - Mycosis fungoides is a type of **cutaneous T-cell lymphoma**, primarily involving **CD4+ T cells** which infiltrate the skin [1][2]. - The disease is characterized by **pleomorphic** skin lesions caused by **malignant T-cell proliferation** [3]. *K Cells (not primarily involved in mycosis fungoides)* - K Cells are involved in **immunological responses** but are not specifically linked to mycosis fungoides. - They do not play a primary role in **cutaneous lymphoproliferative disorders**. *B Cells (involved in humoral immunity)* - B Cells are mainly responsible for **antibody production**, which is not the primary mechanism in mycosis fungoides. - The condition involves **T cell malignancy**, rather than abnormalities in B cell function. *NK Cells (part of innate immunity)* - NK Cells are important for **innate immunity** and target viral and tumor cells but are not primarily involved in this lymphoma. - Mycosis fungoides is characterized by **T cell-mediated responses**, not NK cell activity. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 613-614. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Skin, p. 1162. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 564-565.
Question 2: Identify the diagnosis based on the dermatology immunofluorescence (IF) image provided.
- A. Pemphigus vulgaris
- B. Pemphigus foliaceus
- C. Bullous pemphigoid
- D. Dermatitis herpetiformis (Correct Answer)
Explanation: ***Dermatitis herpetiformis*** - The immunofluorescence image shows **granular IgA deposits** at the **dermal papillae region**, which is characteristic of dermatitis herpetiformis. - This condition is strongly associated with **celiac disease** and presents with intensely pruritic papules and vesicles. *Pemphigus vulgaris* - Immunofluorescence in pemphigus vulgaris typically shows a **fishnet pattern** of IgG deposits throughout the **epidermis**, reflecting antibodies against desmoglein 3 and 1. - This pattern is an intercellular deposition, not granular at the dermal papillae. *Pemphigus foliaceus* - Similar to pemphigus vulgaris, pemphigus foliaceus also exhibits **intercellular IgG deposits** in the epidermis, but it is usually more superficial, targeting desmoglein 1. - The image does not show this intercellular epidermal staining. *Bullous pemphigoid* - Bullous pemphigoid is characterized by **linear IgG and C3 deposits along the dermal-epidermal junction** (basement membrane zone). - The image distinctly shows granular IgA, not linear IgG/C3, and specifically in the dermal papillae.
Question 3: Which of the following statements about Mycosis fungoides is not true?
- A. It has an indolent course but is not easily amenable to treatment. (Correct Answer)
- B. Pautrier's microabscesses are characteristic histopathological features
- C. It is the most common form of cutaneous lymphoma
- D. Erythroderma is seen and it spreads to peripheral circulation
Explanation: ***It has an indolent course but is not easily amenable to treatment.*** - While mycosis fungoides generally has an **indolent course**, it is often highly **amenable to treatment**, especially in its early stages with topical therapies. - Various treatment modalities, including **topical steroids**, **phototherapy**, and **topical chemotherapy**, can effectively manage symptoms and achieve remission. *It is the most common form of cutaneous lymphoma* - Mycosis fungoides is indeed the **most common type of primary cutaneous T-cell lymphoma**, accounting for approximately half of all cases. - This characteristic makes it a significant entity in dermatologic oncology. *Pautrier's microabscesses are characteristic histopathological features* - **Pautrier's microabscesses**, which are collections of atypical lymphocytes within the epidermis, are a **pathognomonic microscopic finding** in mycosis fungoides. - Their presence helps in the histopathological diagnosis of the disease. *Erythroderma seen and spreads to peripheral circulation* - When mycosis fungoides progresses to involve diffuse erythroderma and significant atypical T-cells are found in the peripheral blood, the condition is specifically termed **Sézary syndrome**. - This systemic involvement indicates a more advanced and aggressive form of the disease.
Question 4: Which marker is commonly associated with positivity in follicular lymphoma?
- A. Bcl-1
- B. Bcl-6
- C. Bcl-2 (Correct Answer)
- D. None of the options
Explanation: ***Bcl-2*** - **Follicular lymphoma** is characterized by the overexpression of the **Bcl-2 protein**, which inhibits apoptosis, leading to the survival of malignant B cells [1][3]. - The **Bcl-2 gene** is often involved in the **t(14;18)** chromosomal translocation, which is a hallmark of this lymphoma [1][3][4]. *Bcl-6* - Although **Bcl-6** can be expressed in some lymphomas, it is primarily associated with **diffuse large B-cell lymphoma**, not follicular lymphoma. - **Bcl-6** is involved in **germinal center formation** and its positivity does not indicate follicular lymphoma specifically. *Bcl-1* - **Bcl-1** (also known as **CCND1**) is primarily associated with **mantle cell lymphoma** and is not a characteristic marker for follicular lymphoma. - It is linked to the **t(11;14)** translocation, which is distinct from the genetic alterations seen in follicular lymphoma. *None of the above* - This option is incorrect as **Bcl-2 positivity** is definitive for follicular lymphoma [2]. - The presence of other markers like **Bcl-6** or **Bcl-1** does not negate the expression of Bcl-2 in this lymphoma type. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 602-604. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 604. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 561-562. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 310-311.
Question 5: A 22-year-old woman presents with painless cervical lymphadenopathy, night sweats, and generalized pruritus. An enlarged cervical lymph node is removed for diagnosis, which shows broad bands of fibrosis on cut surface and histologically contains a mixture of cells, including lymphocytes, histiocytes, eosinophils, plasma cells, and scattered large cells with prominent nucleoli. Which of the following is the most likely condition?
- A. Cat-scratch disease
- B. Hodgkin disease (Correct Answer)
- C. Reactive non-specific lymphadenitis
- D. Non-Hodgkin lymphoma
Explanation: ***Hodgkin disease*** - The presence of **painless cervical lymphadenopathy**, night sweats, and **generalized pruritus** is classical for Hodgkin lymphoma [3][4]. - Histological findings of **broad bands of fibrosis** and a mixture of cell types including **Reed-Sternberg cells** confirm the diagnosis [1][2][3]. *Non-Hodgkin lymphoma* - Typically presents with **rapidly enlarging lymph nodes** and is often associated with extra-nodal involvement, differing from the features in this case [3]. - Histology usually shows a more homogeneous population of **malignant lymphoid cells**, not the fibrotic bands seen in Hodgkin disease [3]. *Reactive non-specific lymphadenitis* - This condition is characterized by **hyperplastic lymphoid tissue** and **neutrophilic infiltration**, often following infection, which does not match the findings. - It typically lacks the distinct cell types and fibrosis observed in the biopsy of Hodgkin disease. *Cat-scratch disease* - Caused by *Bartonella henselae*, it often presents with **granulomatous lymphadenopathy** rather than the mixed cellular response seen in Hodgkin disease. - Symptoms usually include a history of cat exposure and may present with **fever, fatigue**, and localized lymphadenopathy, not generalized pruritus. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 616-618. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 616. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 614-616. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 618.
Question 6: Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen
- A. A-3, B-4, C-2, D-1
- B. A-1, B-4, C-3, D-2 (Correct Answer)
- C. A-4, B-2, C-3, D-1
- D. A-2, B-4, C-3, D-1
Explanation: **A-1, B-4, C-3, D-2** - **Caplan syndrome** was first described in **coal workers** with **rheumatoid arthritis** and progressive massive fibrosis. - **Asbestosis** is often associated with **pleural effusion**, which can be benign or malignant. - **Mesothelioma** typically involves the **lower lobes** of the lungs, specifically the pleura, and is strongly linked to asbestos exposure. - **Sarcoidosis** is characterized by **non-caseating granulomas**, which have a predilection for the **upper lobes** of the lungs. *A-3, B-4, C-2, D-1* - This option incorrectly states that Caplan syndrome involves the lower lobe; **Caplan syndrome** is defined by the presence of large nodules in the lungs of coal workers with rheumatoid arthritis, and their specific lobar distribution is not a defining characteristic. - This option incorrectly states that Mesothelioma has an upper lobe predominance; **Mesothelioma** is a pleural malignancy and typically involves the **lower lobes**, extending along the pleura. *A-4, B-2, C-3, D-1* - This option incorrectly associates Caplan syndrome with pleural effusion; **Caplan syndrome** manifests as rheumatoid nodules in the lungs, not primarily pleural effusion. - This option incorrectly states that Asbestosis has an upper lobe predominance; **Asbestosis** predominantly affects the **lower lobes** of the lungs, causing interstitial fibrosis. *A-2, B-4, C-3, D-1* - This option incorrectly states that Caplan syndrome has an upper lobe predominance; the defining feature of **Caplan syndrome** is the combination of rheumatoid arthritis and pneumoconiosis, not specific lobar involvement. - This option correctly identifies pleural effusion with asbestosis and lower lobe involvement with mesothelioma, but **Caplan syndrome** is not characterized by upper lobe predominance.
Question 7: Pautrier's microabscess is a histological feature of which disease?
- A. Sarcoidosis
- B. Tuberculosis
- C. Mycosis fungoides (Correct Answer)
- D. Pityriasis lichenoides chronica
Explanation: ***Mycosis fungoides*** - **Pautrier's microabscesses** (intraepidermal collections of neoplastic T-lymphocytes) are a characteristic histological hallmark of **mycosis fungoides**, a cutaneous T-cell lymphoma [1]. - These collections are typically seen in the **epidermis**, especially in the patch and plaque stages of the disease, reflecting the **epidermotropism** of the malignant T-cells [1]. *Sarcoidosis* - Characterized by **non-caseating granulomas** in various organs, including the skin. - **Pautrier's microabscesses** are not a feature of sarcoidosis. *Tuberculosis* - Identified by the presence of **caseating granulomas** composed of epithelioid cells, lymphocytes, and Langerhans giant cells. - It does not involve the formation of **intraepidermal microabscesses** of lymphocytes. *Pityriasis lichenoides chronica* - A benign inflammatory skin condition characterized by a **lymphocytic vasculitis** and interface dermatitis. - Histology shows a **wedge-shaped inflammatory infiltrate** in the dermis with interface changes, but not Pautrier's microabscesses. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 564-565.
Question 8: What is the dermatological sign associated with carcinoma of the stomach?
- A. Palmoplantar keratoderma
- B. Acquired ichthyosis
- C. Acanthosis Nigrans (Correct Answer)
- D. Acrokeratosis paraneopiastica
Explanation: **Explanation:** **Acanthosis Nigricans (AN)** is the correct answer. While AN is most commonly associated with insulin resistance and obesity (Benign AN), its sudden, severe, and widespread onset in an older individual—often involving the palms (tripe palms) and mucous membranes—is a classic **paraneoplastic syndrome**. **Malignant Acanthosis Nigricans** is most frequently associated with **adenocarcinomas of the gastrointestinal tract**, with **stomach cancer** being the most common (approx. 50-60% of cases). It is thought to be mediated by tumor-secreted growth factors like Transforming Growth Factor-alpha (TGF-α) acting on epidermal EGF receptors. **Analysis of Incorrect Options:** * **A. Palmoplantar Keratoderma (PPK):** While acquired PPK can be paraneoplastic (Howel-Evans Syndrome), it is specifically linked to **Esophageal carcinoma**, not primarily the stomach. * **B. Acquired Ichthyosis:** This sudden onset of "fish-like" scaling in adulthood is most strongly associated with **lymphomas** (specifically Hodgkin’s Lymphoma), rather than gastric malignancies. * **D. Acrokeratosis Paraneoplastica (Bazex Syndrome):** This presents with psoriasiform plaques on acral sites (ears, nose, fingers). It is highly specific for squamous cell carcinomas of the **upper aerodigestive tract** (head, neck, and esophagus). **High-Yield Clinical Pearls for NEET-PG:** * **Tripe Palms:** When AN affects the palms, appearing velvety and rugose, it is 90% predictive of internal malignancy. If seen with AN, think **Stomach CA**; if seen alone, think **Lung CA**. * **Leser-Trélat Sign:** The sudden eruption of multiple Seborrheic Keratoses is another major cutaneous marker for **Gastric Adenocarcinoma**. * **Sister Mary Joseph Nodule:** A palpable nodule at the umbilicus representing metastasis from a pelvic or abdominal malignancy (most commonly Stomach CA).
Question 9: What is the most common malignancy found in Marjolin's ulcer?
- A. Basal cell carcinoma (BCC)
- B. Squamous cell carcinoma (SCC) (Correct Answer)
- C. Malignant fibrous histiocytoma
- D. Malignant melanoma
Explanation: **Explanation:** **Marjolin’s ulcer** refers to a malignancy arising in a setting of chronic inflammation, long-standing scars, or non-healing wounds. The most common underlying cause is a **chronic burn scar**, though it can also occur in chronic osteomyelitis sinuses, venous stasis ulcers, and vaccination scars. 1. **Why Squamous Cell Carcinoma (SCC) is correct:** The chronic irritation and repeated cycles of injury and repair in a scar lead to cellular dysplasia. **Squamous cell carcinoma** is the histological diagnosis in approximately **75-90%** of Marjolin’s ulcer cases. These tumors are typically more aggressive, have a higher rate of metastasis (approx. 30%), and carry a poorer prognosis compared to SCC arising in sun-damaged skin. 2. **Why other options are incorrect:** * **Basal Cell Carcinoma (BCC):** While BCC is the most common skin cancer overall, it is the second most common malignancy in Marjolin’s ulcer (approx. 10%). It is less frequent than SCC in the context of chronic scars. * **Malignant Fibrous Histiocytoma & Malignant Melanoma:** These are extremely rare occurrences in chronic scars. While cases have been reported, they do not represent the "most common" malignancy. **High-Yield Clinical Pearls for NEET-PG:** * **Latency Period:** The average time for malignant transformation is **25–30 years**. * **Characteristic Feature:** A Marjolin’s ulcer is characterized by an everted edge, foul-smelling discharge, and rapid growth in a previously stable scar. * **Lymph Nodes:** Unlike typical SCC, Marjolin’s ulcer often bypasses local lymph nodes or presents with late-stage nodal involvement due to the dense fibrotic scar tissue limiting lymphatic drainage. * **Treatment:** Wide local excision (usually with a 2cm margin) or amputation is the treatment of choice.
Question 10: What is a rodent ulcer?
- A. Infectious ulcer
- B. Hypersensitivity
- C. Basal cell carcinoma (Correct Answer)
- D. Squamous cell carcinoma
Explanation: **Explanation:** **Basal Cell Carcinoma (BCC)** is the correct answer. The term **"Rodent Ulcer"** is a classic clinical description for a specific morphological variant of BCC (nodulo-ulcerative type). It is named so because the ulcer appears as if a rodent has "gnawed" into the skin, characterized by a central depression with **pearly, rolled-out borders** and overlying telangiectasia. * **Why it is correct:** BCC arises from the basal layer of the epidermis. While it is locally invasive and can cause significant tissue destruction (hence "ulcer"), it rarely metastasizes. The "rodent ulcer" typically occurs on sun-exposed areas, particularly the upper face (above the line joining the lobe of the ear to the angle of the mouth). * **Why other options are wrong:** * **Infectious ulcers** (e.g., Syphilitic chancre or Cutaneous Leishmaniasis) have distinct microbiological etiologies and different edge characteristics. * **Hypersensitivity** reactions usually present as dermatitis, wheals, or target lesions (Erythema Multiforme), not as chronic destructive ulcers. * **Squamous Cell Carcinoma (SCC)** typically presents as an ulcer with **everted edges** and has a much higher potential for lymphatic metastasis compared to BCC. **High-Yield Clinical Pearls for NEET-PG:** * **Most common** skin cancer globally: Basal Cell Carcinoma. * **Commonest site:** Nose (specifically the ala). * **Histopathology:** Shows "Peripheral Palisading" of nuclei and "Retraction Artifacts." * **Risk Factor:** Chronic UV light exposure and mutations in the **PTCH gene** (Gorlin Syndrome). * **Treatment of choice:** Surgical excision or Mohs Micrographic Surgery (for high-risk sites).
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