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Multiple hamartoma syndrome: clinicoradiological evaluation and histopathological correlation with brief review of literature.

Monga E, Gupta PK, Munshi A, Agarwal S - Indian J Dermatol (2014)

Bottom Line: Cowden syndrome (CS) or multiple hamartoma syndromes (MHSs) is an uncommon condition and characterized by mucocutaneous lesions which may be associated with the lesions of breast, thyroid, gastrointestinal tract, skin, and often of central nervous system.We are reporting a case of female patient who presented with multiple mucocutaneous papilloma associated with involvement of multiple organs and systems.Being a rare case, we are discussing MHS with its clinicoradiological and histopathological correlation along with brief review of literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis, Imaging and Interventional Radiology, N.S.C.B. Subharti Medical College, Swami Vivekananda Subharti University, Subhartipuram, NH-58, Meerut, Uttar Pradesh, India.

ABSTRACT
Cowden syndrome (CS) or multiple hamartoma syndromes (MHSs) is an uncommon condition and characterized by mucocutaneous lesions which may be associated with the lesions of breast, thyroid, gastrointestinal tract, skin, and often of central nervous system. A thorough evaluation of Cowden's disease is essential due to increased risk of malignancy in an organ or system of the affected patients. We are reporting a case of female patient who presented with multiple mucocutaneous papilloma associated with involvement of multiple organs and systems. The diagnosis was confirmed by multimodality diagnostic approach. This rare entity has not being reviewed in Indian literature till date. Being a rare case, we are discussing MHS with its clinicoradiological and histopathological correlation along with brief review of literature.

No MeSH data available.


Related in: MedlinePlus

Papules and furrowing on tongue
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Figure 2: Papules and furrowing on tongue

Mentions: Clinical examination revealed multiple tiny (1-2 mm) nodules on forehead, nose, and maxillary region with pallor (anemia). The right half of lower lip showed cluster of papillomatous lesions [Figure 1]. There were keratotic papules on tongue and palatal surface along with thickening and furrowing of tongue [Figure 2]. Soft palpable nodules on palm and acral keratoses were also seen [Figure 3]. Both breasts and lobes of thyroid gland also showed small and palpable multiple nodules. The nodules in the breast were mobile without any change on overlying skin or nipple areola complex. Other systemic examination was unremarkable and no evidence of any lymphadenopathy was shown.


Multiple hamartoma syndrome: clinicoradiological evaluation and histopathological correlation with brief review of literature.

Monga E, Gupta PK, Munshi A, Agarwal S - Indian J Dermatol (2014)

Papules and furrowing on tongue
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4248500&req=5

Figure 2: Papules and furrowing on tongue
Mentions: Clinical examination revealed multiple tiny (1-2 mm) nodules on forehead, nose, and maxillary region with pallor (anemia). The right half of lower lip showed cluster of papillomatous lesions [Figure 1]. There were keratotic papules on tongue and palatal surface along with thickening and furrowing of tongue [Figure 2]. Soft palpable nodules on palm and acral keratoses were also seen [Figure 3]. Both breasts and lobes of thyroid gland also showed small and palpable multiple nodules. The nodules in the breast were mobile without any change on overlying skin or nipple areola complex. Other systemic examination was unremarkable and no evidence of any lymphadenopathy was shown.

Bottom Line: Cowden syndrome (CS) or multiple hamartoma syndromes (MHSs) is an uncommon condition and characterized by mucocutaneous lesions which may be associated with the lesions of breast, thyroid, gastrointestinal tract, skin, and often of central nervous system.We are reporting a case of female patient who presented with multiple mucocutaneous papilloma associated with involvement of multiple organs and systems.Being a rare case, we are discussing MHS with its clinicoradiological and histopathological correlation along with brief review of literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis, Imaging and Interventional Radiology, N.S.C.B. Subharti Medical College, Swami Vivekananda Subharti University, Subhartipuram, NH-58, Meerut, Uttar Pradesh, India.

ABSTRACT
Cowden syndrome (CS) or multiple hamartoma syndromes (MHSs) is an uncommon condition and characterized by mucocutaneous lesions which may be associated with the lesions of breast, thyroid, gastrointestinal tract, skin, and often of central nervous system. A thorough evaluation of Cowden's disease is essential due to increased risk of malignancy in an organ or system of the affected patients. We are reporting a case of female patient who presented with multiple mucocutaneous papilloma associated with involvement of multiple organs and systems. The diagnosis was confirmed by multimodality diagnostic approach. This rare entity has not being reviewed in Indian literature till date. Being a rare case, we are discussing MHS with its clinicoradiological and histopathological correlation along with brief review of literature.

No MeSH data available.


Related in: MedlinePlus